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  • Author or Editor: Hugh Garton x
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Editorial

Tethered cord release

Joseph H. Piatt Jr.

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Wajd N. Al-Holou, Cormac O. Maher, Karin M. Muraszko and Hugh J. L. Garton

Object

The authors reviewed their experience with pediatric pineal cysts to define the natural history and clinical relevance of this common intracranial finding.

Methods

The authors identified all patients with pineal cysts who had been clinically evaluated at their institution over an 11.5-year interval and were < 25 years of age at the time of diagnosis. All inclusion criteria were met in 106 patients, and included repeated MR imaging as well as repeated clinical evaluation over at least a 6-month interval.

Results

The mean age at diagnosis was 11.7 ± 7.2 years. Forty-two patients were male and 64 were female. On follow-up MR imaging evaluation at a mean interval of 3.0 years from the initial study, 98 pineal cysts had no increase in size and no change in imaging appearance. Six pineal cysts increased in size and 2 others had a change in imaging characteristics without associated growth. Younger age was associated with cyst change or growth on follow-up imaging (p = 0.02). The mean age of patients with cysts that changed or grew was 5.5 years, and the mean age of patients with stable pineal cysts was 12.2 years. Initial cyst size and appearance on MR imaging were not significant predictors of growth or change in imaging appearance at follow-up. Similarly, the patient's sex was not a significant predictor of growth or change in imaging characteristics.

Conclusions

Follow-up imaging and neurosurgical evaluation may be considered optional in older children with pineal cysts.

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Jennifer Strahle, Béla J. Selzer, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher

Object

The authors investigated the effect of a tablet computer on performance-level settings of a programmable shunt valve.

Methods

Magnetic field strength near the tablet computer with and without a cover was recorded at distances between 0 and 100 mm. Programmable valves were exposed to the tablet device at distances of less than 1 cm, 1–2.5 cm, 2.5–5 cm, 5–10 cm, and greater than 10 cm. For each distance tested, the valves were exposed 100 times to the tablet with the cover, resulting in 500 total valve exposures. The tablet alone, without the cover, was also tested at distances of less than 1 cm for 30 valve exposures. Changes in valve performance-level settings were recorded.

Results

The maximum recorded magnetic flux density of a tablet with a cover was 17.0 mT, and the maximum recorded magnetic flux density of the tablet alone was 7.6 mT. In 100 exposures at distances between 0 and 1 cm, 58% of valves had different settings following exposure. At distances greater than 1 cm but less than 2.5 cm, 5% of valves in 100 exposures had setting changes. Only a single setting change was noted in 100 exposures at distances greater than 2.5 cm but less than 5 cm. No setting changes were noted at distances greater than 5 cm, including 100 exposures between 5 and 10 cm, and 100 exposures of more than 10 cm. For the 30 valve exposures to the tablet without a cover, 20 valve performance-level changes (67%) were noted.

Conclusions

Based on these results, exposure to tablet devices may alter programmable shunt valve settings.

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Wajd N. Al-Holou, Karin M. Muraszko, Hugh J. Garton, Steven R. Buchman and Cormac O. Maher

Object

After primary repair of a myelomeningocele or a lipomyelomeningocele, patients can present with symptoms of secondary tethered cord syndrome (TCS). After surgical untethering, a small percentage of these patients can present with multiple repeat TCS. In patients presenting with secondary or multiple repeat TCS, the role as well the expected outcomes of surgical untethering are not well defined.

Methods

Eighty-four patients who underwent spinal cord untethering after at least 1 primary repair were retrospectively evaluated using scaled and subjective outcome measures at short-term and long-term follow-up visits. Outcomes were analyzed for predictive measures using multivariate logistic regression.

Results

Surgical untethering was performed in 66 patients with myelomeningoceles and 18 patients with lipomyelomeningoceles. Fourteen patients underwent multiple repeat spinal cord untethering. Patients were followed up for an average of 6.2 years. Most patients had stability of function postoperatively. Motor function and weakness improved in 7 and 16% of patients at 6 months, respectively, and 6 and 19% of patients at long-term follow-up evaluation, respectively. Of the patients who presented with back pain, 75% had improvement in symptoms at 6 months postoperatively. Younger age at untethering was significantly associated with worse long-term neurological outcomes. The number of previous untethering procedures, original diagnosis, sex, anatomical level, and degree of untethering had no effect on surgical outcomes.

Conclusions

Patients presenting with secondary or multiple repeat TCS may benefit from surgical untethering.

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D. Andrew Wilkinson, Kyle Johnson, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher

OBJECTIVE

The goal of this analysis was to define temporal and geographic trends in the surgical treatment of Chiari malformation Type I (CM-I) in a large, privately insured health care network.

METHODS

The authors examined de-identified insurance claims data from a large, privately insured health care network of over 58 million beneficiaries throughout the United States for the period between 2001 and 2014 for all patients undergoing surgical treatment of CM-I. Using a combination of International Classification of Diseases (ICD) diagnosis codes and Current Procedural Terminology (CPT) codes, the authors identified CM-I and associated diagnoses and procedures over a 14-year period, highlighting temporal and geographic trends in the performance of CM-I decompression (CMD) surgery as well as commonly associated procedures.

RESULTS

There were 2434 surgical procedures performed for CMD among the beneficiaries during the 14-year interval; 34% were performed in patients younger than 20 years of age. The rate of CMD increased 51% from the first half to the second half of the study period among younger patients (p < 0.001) and increased 28% among adult patients between 20 and 65 years of age (p < 0.001). A large sex difference was noted among adult patients; 78% of adult patients undergoing CMD were female compared with only 53% of the children. Pediatric patients undergoing CMD were more likely to be white with a higher household net worth. Regional variability was identified among rates of CMD as well. The average annual rate of surgery ranged from 0.8 surgeries per 100,000 insured person-years in the Pacific census division to 2.0 surgeries per 100,000 insured person-years in the East South Central census division.

CONCLUSIONS

Analysis of a large nationwide health care network showed recently increasing rates of CMD in children and adults over the past 14 years.

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Jeffrey L. Nadel, D. Andrew Wilkinson, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher

OBJECTIVE

The goal of this study was to determine the rates of screening and surgery for foramen magnum stenosis in children with achondroplasia in a large, privately insured healthcare network.

METHODS

Rates of screening and surgery for foramen magnum stenosis in children with achondroplasia were determined using de-identified insurance claims data from a large, privately insured healthcare network of over 58 million beneficiaries across the United States between 2001 and 2014. Cases of achondroplasia and screening and surgery claims were identified using a combination of International Classification of Diseases diagnosis codes and Current Procedural Terminology codes. American Academy of Pediatrics (AAP) practice guidelines were used to determine screening trends.

RESULTS

The search yielded 3577 children age 19 years or younger with achondroplasia. Of them, 236 met criteria for inclusion in the screening analysis. Among the screening cohort, 41.9% received some form of screening for foramen magnum stenosis, whereas 13.9% of patients were fully and appropriately screened according to the 2005 guidelines from the AAP. The screening rate significantly increased after the issuance of the AAP guidelines. Among all children in the cohort, 25 underwent cervicomedullary decompression for foramen magnum stenosis. The incidence rate of undergoing cervicomedullary decompression was highest in infancy (28 per 1000 patient-years) and decreased with age (5 per 1000 patient-years for all other ages combined).

CONCLUSIONS

Children with achondroplasia continue to be underscreened for foramen magnum stenosis, although screening rates have improved since the release of the 2005 AAP surveillance guidelines. The incidence of surgery was highest in infants and decreased with age.

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Javier Márquez and Mónica Rivero

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Wajd N. Al-Holou, Hugh J. L. Garton, Karin M. Muraszko, Mohannad Ibrahim and Cormac O. Maher

Object

Pineal cysts are a frequent incidental finding on intracranial imaging. In adults, the prevalence of pineal cysts is estimated to be 1.1–4.3%. However, the prevalence is not well established in younger patients.

Methods

The authors retrospectively reviewed a consecutive series of 14,516 patients 25 years of age and younger, who underwent brain MR imaging at a single institution over an 11-year period. In patients identified with pineal cysts, the authors analyzed the images according to cyst size, signal characteristics, enhancement pattern, and evidence of local mass effect. Patient characteristics including demographics and other intracranial diagnoses were collected in the pineal cyst population and compared with a randomly selected age- and sex-matched control patient population. The data were evaluated using univariate and multivariate logistic regression, linear regression, and ANOVA.

Results

The authors identified 288 pineal region cysts (2.0%). The prevalence of pineal cysts was higher in female (2.4%) than in male patients (1.5%; p < 0.001). Pineal cysts were identified in patients of all ages, with an increased prevalence found in older patients (p < 0.001). Pineal cyst size was similar for all age and sex groups.

Conclusions

Pineal cysts are common in the pediatric population, with an increased prevalence in girls and in older patients.

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Brandon W. Smith, Jennifer Strahle, Erick Kazarian, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher

OBJECT

It is unclear if there is a relationship between Chiari malformation Type I (CM-I) and body mass index (BMI). The aim of this study was to identify the relationship between BMI and cerebellar tonsil position in a random sample of people.

METHODS

Cerebellar tonsil position in 2400 subjects from a cohort of patients undergoing MRI was measured. Three hundred patients were randomly selected from each of 8 age groups (from 0 to 80 years). A subject was then excluded if he or she had a posterior fossa mass or previous posterior fossa decompression or if height and weight information within 1 year of MRI was not recorded in the electronic medical record.

RESULTS

There were 1310 subjects (54.6%) with BMI records from within 1 year of the measured scan. Of these subjects, 534 (40.8%) were male and 776 (59.2%) were female. The average BMI of the group was 26.4 kg/m2, and the average tonsil position was 0.87 mm above the level of the foramen magnum. There were 46 subjects (3.5%) with a tonsil position ≥ 5 mm below the level of the foramen magnum. In the group as a whole, there was no correlation (R2 = 0.004) between BMI and cerebellar tonsil position.

CONCLUSIONS

In this examination of 1310 subjects undergoing MRI for any reason, there was no relationship between BMI and the level of the cerebellar tonsils or the diagnosis of CM-I on imaging.

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Brandon W. Smith, Jennifer Strahle, J. Rajiv Bapuraj, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher

Object

Prior attempts to define normal cerebellar tonsil position have been limited by small numbers of patients precluding analysis of normal distribution by age group. The authors' objective in the present study was to analyze cerebellar tonsil location in every age range.

Methods

Two thousand four hundred patients were randomly selected from a database of 62,533 consecutive patients undergoing MRI and were organized into 8 age groups. Magnetic resonance images were directly examined for tonsil location, morphology, and other features. Patients with a history or imaging findings of posterior fossa abnormalities unrelated to Chiari malformation (CM) were excluded from analysis. The caudal extent of the cerebellar tonsils was measured at the midsagittal and lowest parasagittal positions.

Results

The mean tonsil height decreased slightly with advancing age into young adulthood and increased with advancing age in the adult age range. An increasing age in the adult age range was associated with a decreased likelihood of a tonsil position 5 mm or more below the foramen magnum (p = 0.0004). In general, the lowest tonsil position in each age group was normally distributed. Patients with pegged morphology were more likely to have a tonsil location at least 5 mm below the foramen magnum (85%), as compared with those having intermediate (38%) or rounded (2%) morphology (p < 0.0001). Female sex was associated with a lower mean tonsil position (p < 0.0001). Patients with a lower tonsil position also tended to have an asymmetrical tonsil position, usually lower on the right (p < 0.0001).

Conclusions

Cerebellar tonsil position follows an essentially normal distribution and varies significantly by age. This finding has implications for advancing our understanding of CM.